Ann Rheum Dis

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Ann Rheum Dis 1998;57:296-302 ( May )

Extended reports

Heart involvement in systemic sclerosis: an ultrasonic tissue characterisation study Clodoveo Ferri,a Vitantonio Di Bello,a Antonella Martini,a Davide Giorgi,a Franca A A Storino,a Massimiliano Bianchi,a Alessio Bertini,a Marco Paterni,b Costantino Giusti,a Giampiero Paseroa

a Rheumatology Unit, Department of Internal Medicine, University of Pisa, Italy, b Institute of Clinical Physiology, CNR, Pisa, Italy

Correspondence to: Professor C Ferri, Rheumatology Unit, Department of Internal Medicine, Via Roma 67, 56100 Pisa, Italy.

Accepted for publication 10 March 1998

BACKGROUND---Clinicoepidemiological findings indicate that symptomatic heart involvement in patients with systemic sclerosis (SSc) predicts a very poor prognosis. At necropsy studies, SSc heart involvement without significant coronary lesions is characterised by patchy myocyte necrosis and contraction band necrosis with collagen replacement leading to myocardial fibrosis. There is a discrepancy between the frequency of clinically evident myocardial disease (25%) and autoptical myocardial fibrosis (81%).
OBJECTIVE---The aim of this study was to detect preclinical myocardial alterations in SSc patients by ultrasonic videodensitometric analysis.
METHODS---Thirty five SSc patients (three male, aged 48.6 (11) SD years, range 22-65) with normal ventricular function and 25 age and sex matched healthy controls were studied. All patients had a negative maximal exercise stress; in all cases arterial hypertension, renal involvement, and diabetes were excluded. Echocardiographic images were digitised by a real time videodigitiser (Tomtec Imaging Systems). Quantitative texture analysis was performed on data from the septum and the posterior wall, obtaining mean gray level histogram (MGL) at both end-diastole (d) and end-systole (s). The cyclic variation index (CVI), was calculated according to the formula ((MGLd-MGLs)/MGLd) × 100. Left ventricular mass (LVM), body surface corrected, was calculated according to Penn convention.
RESULTS---Comparable systolic and diastolic blood pressure, LVM, diastolic and systolic function were recorded in both SSc patients and controls. In contrast, in SSc patients the CVI, which is the expression of the intrinsic myocardial structural function, was significantly lower than in controls (septum: -18 (28)% v 35 (10)%, p<0.0001; and posterior wall: -13 (32)% v 50 (20)%, p<0.0001). Changes in cyclic echo amplitude, probably related to myocardial fibrosis, were detected in the large majority of SSc patients (88%).
CONCLUSIONS---Ultrasonic videodensitometric analysis represents a non-invasive, feasible method that can detect early myocardial changes in SSc patients, which could be related to both fibrosis and microcirculatory abnormalities. Their potential evolution towards ventricular dysfunction and their link with cardiac sudden death, because of severe conduction system or rhythm disturbancies, should be further investigated.

Keywords: systemic sclerosis; heart involvement; echocardiography; ultrasonic; videodensitometry


© 1998 by Annals of the Rheumatic Diseases



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